目的 探索儿童肝炎相关再生障碍性贫血（HAAA）临床特征。 方法 选取212例再生障碍性贫血（AA）患儿为研究对象，根据病因分为HAAA组和非HAAA组，以23例HAAA组为研究组，按照年龄、性别和再生障碍性贫血严重程度1∶5比例匹配同期115例非HAAA组患儿为对照组，分析23例HAAA组的临床特征、治疗方案及病情转归，并与对照组的临床特征、实验室检查结果相比较。 结果 HAAA患儿中男11例，女12例，中位年龄为6岁3个月（1岁4个月 ~ 12岁）。23例患儿发病过程均为急性肝炎起病，经治疗肝功能逐渐好转后外周血象逐渐出现包括血小板在内的两系或三系下降，其中14例（14/23，60.9%）为极重型再生障碍性贫血，7例（7/23，30.4%）为重型再生障碍性贫血，2例（2/23，8.7%）为非重型再生障碍性贫血。肝炎发生至确诊为再生障碍性贫血中位间隔时间为56 d（10 ~ 157 d）。23例（23/23，100%）患儿均有黄疸，1例（1/23，4.3%）患儿基因检查确诊为X-连锁淋巴增生性疾病2型，5例（5/23，21.7%）患儿行肝穿刺活检示药物性肝炎/化学性肝损伤。HAAA组患儿CD4⁺细胞比例（1.2 ± 0.3）和CD4⁺/CD8 ⁺比值（-0.2 ± 0.4）与对照组患儿的CD4⁺细胞比例（1.5 ± 0.1）和CD4⁺/CD8⁺比值（0.1 ± 0.2）相比明显下降（P < 0.05）。3例（3/23，13.0%）患儿进行免疫抑制治疗（IST）好转，18例（18/23，78.3%）接受造血干细胞移植（HSCT），2例（2/23，8.7%）非重型再障仅应用甲泼尼龙琥珀酸钠及复方皂矾丸治疗，随访至2023年12月所有患儿均存活。 结论 儿童HAAA病情危重，预后差，多为重型或极重型再生障碍性贫血，多发生在肝炎恢复期，CD4⁺细胞减少和CD4⁺/CD8⁺比值倒置或为HAAA发生预警因子，免疫抑制治疗和造血干细胞移植可有效改善预后。

Objective To investigate the clinical characteristics of hepatitis?associated aplastic anemia （HAAA） in pediatric patients. Methods A retrospective study was conducted on 212 children with aplastic anemia （AA） who were hospitalized at Henan Children's Hospital from September 2014 to February 2023. The patients were categorized into two groups based on etiology： the HAAA group and the non?HAAA group. The study group consisted of 23 patients in the HAAA group， while a control group of 115 children without HAAA was matched in a 1∶5 ratio based on age， sex， and severity of aplastic anemia. The clinical characteristics， treatment regimens， and outcomes of the 23 patients with HAAA were analyzed and compared with those of the control group comprising 115 patients. Results Among the 23 children with HAAA， there were 11 males and 12 females， with a median age of 6 years and 3 months （ranging from 1 year and 4 months to 12 years old）. The onset of aplastic anemia in all HAAA children occurred after the initial presentation of acute hepatitis. Following gradual improvement in liver function， peripheral blood images showed a progressive decline by two or three lines， including platelets. Among these cases， very severe aplastic anemia was observed in 14 patients （60.9%）， severe aplastic anemia in 7 patients （30.4%）， and non?severe aplastic anemia in 2 patients （8.7%）. The median interval between hepatitis onset and diagnosis of aplastic anemia was found to be 56 days （range：10 ~ 157 days）. All 23 pediatric patients with HAAA presented with acute icteric hepatitis， accounting for 100% of the cohort. One patient （4.3%） was genetically diagnosed with X?linked lymphoproliferative disease type 2， while liver biopsy revealed drug?induced hepatitis/chemical liver injury in five patients （21.7%）. Compared to the control group， HAAA patients exhibited significantly lower levels of CD4⁺ cells ［（1.2 ± 0.3） vs. （1.5 ± 0.1）］ and CD4⁺/CD8⁺ ratios ［（-0.2 ± 0.4） vs. （0.1 ± 0.2）］ （P < 0.05）. Three patients received immunosuppressive therapy （IST）， 18 underwent hematopoietic stem cell transplantation （HSCT）， and two non?severe cases were treated with methylprednisolone sodium succinate and compound Zapoan pill； all patients survived. Conclusions Children with HAAA present a critical condition and exhibit a poor prognosis， predominantly manifesting as severe or extremely severe aplastic anemia during the recovery phase of hepatitis. Reduction in CD4⁺ cell count and inversion of CD4⁺/CD8⁺ ratio may serve as early warning indicators for HAAA. Effective improvement in prognosis can be achieved through immunosuppressive therapy and hematopoietic stem cell transplantation.